[13C]valine metabolism in methylmalonicacidemia using nuclear magnetic resonance: propionate as an obligate intermediate

K. Tanaka, Ian M Armitage, H. S. Ramsdell

Research output: Contribution to journalArticle

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Abstract

13C] and [α,β 13C]valine were administered sequentially to a patient with methylmalonicacidemia to clarify the metabolic pathway of valine from methylmalonic acid semialdehyde to methylmalonyl CoA. Methylmalonic acid was isolated from multiple urine samples, purified, and analyzed by 13C nuclear magnetic resonance spectroscopy. Contrary to the widely accepted view, the results show unequivocally that methylmalonic acid semialdehyde is decarboxylated to propionate before conversion to methylmalonyl CoA.

Original languageEnglish (US)
Pages (from-to)3692-3696
Number of pages5
JournalProceedings of the National Academy of Sciences of the United States of America
Volume72
Issue number9
DOIs
StatePublished - Jan 1 1975
Externally publishedYes

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